CASE REPORT
A Rare Combination: Multiple Endocrine Neoplasia Type 1 and Follicular Thyroid Carcinoma
Nadir Bir Kombinasyon: Multipl Endokrin Neoplazi Tip 1 ve Foliküler Tiroid Karsinomu
Received Date : 07 Jul 2019
Accepted Date : 02 Jan 2020
Available Online : 24 Jan 2020
Ahmet GÖRGEL, Sacit Nuri GÖRGEL*, Mustafa DEMİRPENÇE, Mitat BAHÇECİ
Atatürk Training and Research Hospital, Department of Endocrinology and Metabolism, İzmir, Turkey
*Atatürk Training and Research Hospital, Department of Urology, İzmir, Turkey
Doi: 10.25179/tjem.2019-70603 - Makale Dili: EN
Turk J Endocrinol Metab 2020;24:87-91
ABSTRACT
Multiple endocrine neoplasia Type 1 (MEN-1) is an inherited
syndrome characterized by the development of endocrine
tumors of the pancreas, parathyroid, and pituitary glands.
Mesenchymal tumors and adrenal neoplasms might also accompany
this syndrome. However, the syndrome is rarely
associated with thyroid tumors in contrary to the multiple
endocrine neoplasia Type 2 that includes medullary thyroid
carcinoma. This case study presents a 44-year-old woman
who was diagnosed with MEN-1 on the basis of her clinical
characteristics, laboratory data, and the presence of endocrine
tumors. Follicular thyroid carcinoma was detected
in the patient when she was being operated for nodular goiter,
12 years ago. We report this rare case which is likely
the third case in the available scientific literature.
Keywords: Multiple endocrine neoplasia Type 1; follicular thyroid carcinoma, nephrolithiasis; hypoglycemia; acromegaly
ÖZET
Multipl endokrin neoplazi Tip 1 (MEN-1) pankreas, paratiroid
ve piuiter bezlerde endokrin tümörlerin gelişimiyle karakterize,
kalıtımsal bir sendromdur. Mezenkimal tümörler
ve adrenal neoplaziler de bu sendroma eşlik edebilir. Bununla
birlikte, medüller tiroid karsinomu içeren multipl endokrin
neoplazi Tip 2’nin aksine, bu sendrom, tiroid
tümörleri ile nadiren ilişkilidir. Bu çalışmada, endokrin tümörlerin
varlığı, laboratuvar verileri ve karakteristik klinik
özelliklerin ışığında MEN-1 tanısı konan 44 yaşındaki bir
kadın olgu sunulmaktadır. Olgu nodüler guatr için 12 yıl
önce opere edildiğinde foliküler tiroid karsinomu tespit edilmişti.
Mevcut bilimsel literatürde, muhtemelen 3. vaka olan
bu ender olguyu bildirmekteyiz.
Anahtar Kelimeler: Multipl endokrin neoplazi tip 1; folliküler tiroid karsinomu; nefrolitiazis; hipoglisemi; akromegali
KAYNAKLAR
- Chandrasekharappa SC, Guru SC, Manickam P, Olufemi SE, Collins FS, Emmert-Buck MR, Debelenko LV, Zhuang Z, Lubensky IA, Liotta LA, Crabtree JS, Wang Y, Roe BA, Weisemann J, Boguski MS, Agarwal SK, Kester MB, Kim YS, Heppner C, Dong Q, Spiegel AM, Burns AL, Marx SJ. Positional cloning of the gene for multiple endocrine neoplasia-type 1. Science. 1997;276:404-407.
- Guo SS, Sawicki MP. Molecular and genetic mechanisms of tumorigenesis in multiple endocrine neoplasia type-1. Mol Endocrinol. 2001;15:1653-1664. [Crossref] [PubMed]
- Brandi ML, Gagel RF, Angeli A, Bilezikian JP, Beck-Peccoz P, Bordi C, Conte-Devolx B, Falchetti A, Gheri RG, Libroia A, Lips CJ, Lombardi G, Mannelli M, Pacini F, Ponder BA, Raue F, Skogseid B, Tamburrano G, Thakker RV, Thompson NW, Tomassetti P, Tonelli F, Wells SA Jr, Marx SJ. Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab. 2001;86:5658-5671. [Crossref] [PubMed]
- Kim YL, Jang YW, Kim JT, Sung SA, Lee TS, Lee WM, Kim HJ. A rare case of primary hyperparathyroidism associated with primary aldosteronism, Hurthle cell thyroid cancer and meningioma. J Korean Med Sci. 2012;27:560-564. [Crossref] [PubMed] [PMC]
- Lodewijk L, Bongers PJ, Kist JW, Conemans EB, de Laat JM, Pieterman CR, van der Horst-Schrivers AN, Jorna C, Hermus AR, Dekkers OM, de Herder WW, Drent ML, Bisschop PH, Havekes B, Rinkes IH, Vriens MR, Valk GD. Thyroid incidentalomas in patients with multiple endocrine neoplasia type 1. Eur J Endocrinol. 2015;172:337-342. [Crossref] [PubMed]
- Liaw CC, Lin JT, Chen TJ. Multiple-hormone-producing islet cell carcinoma: report of a case. Taiwan Yi Xue Hui Za Zhi. 1989;88:722-725.
- Thompson NW. Current concepts in the surgical management of multiple endocrine neoplasia type 1 pancreatic-duodenal disease. Results in the treatment of 40 patients with Zollinger-Ellison syndrome, hypoglycaemia or both. J Intern Med. 1998;243:495-500. [Crossref] [PubMed]
- Lodish MB, Powell AC, Abu-Asab M, Cochran C, Lenz P, Libutti SK, Pingpank JF, Tsokos M, Gorden P. Insulinoma and gastrinoma syndromes from a single intrapancreatic neuroendocrine tumor. J Clin Endocrinol Metab. 2008;93:1123-1128. [Crossref] [PubMed] [PMC]
- Vortmeyer AO, Lubensky IA, Skarulis M, Li G, Moon YW, Park WS, Weil R, Barlow C, Spiegel AM, Marx SJ, Zhuang Z. Multiple endocrine neoplasia type 1: atypical presentation, clinical course, and genetic analysis of multiple tumors. Mod Pathol. 1999;12:919-924. /td>
- Perakakis N, Flohr F, Kayser G, Thomusch O, Parsons L, Billmann F, von Dobschuetz E, Rondot S, Seufert J, Laubner K. Multiple endocrine neoplasia type 1 associated with a new germline Men1 mutation in a family with atypical tumor phenotype. Hormones. 2016;15:113-117. [Crossref] [PubMed]